Recent advances in the understanding of desmoid tumor biology affect therapeutic choices. This series of 19 patients and review of the literature outlines historic perspectives and discusses the options in the management of these locally aggressive tumors. Desmoid tumors tend to grow steadily, regardless of tumor location. However differences in the aggressive nature of these tumors are seen when age and sex distributions are scrutinized. Although recurrence rates are high, excisional therapy is the best first approach. An exception is the case in which tumor excision is either particularly dangerous or likely to result in significant physical handicap. Radiation or drug therapy are most often used with recurrent disease or as an alternative to mutilating surgery. Although many pharmacologic approaches have been advocated, (including antiestrogen therapy, cyclic-AMP, and prostaglandin inhibition), results are anecdotal at best.